Sanfilippo D syndrome: Correction of glucosamine-6-sulphatase deficiency following fibroblast culture in chang's media

The de-O-sulphation of α-linked glucosamine-6-sulphate residues in heparan sulphate requires a specific sulphatase, glucosamine-6-sulphatase, which has been shown to be deficient in tissues of Sanfilippo D, or mucopolysaccharidosis type IIID (MPS IIID), patients. MPS IIID fibroblasts cultured in Basal Eagle's medium supplemented with either fetal calf serum or heat-inactivated fetal calf serum, MDCB or Ultraserg media had residual glucosarnine-6-sulphatase activities towards a heparin-derived trisaccharide substrate, O-(α-N-acetylglucosamine-6-sulphate)-(1→4)-L -O-(α-iduronic acid-2-sulphate)-(1→4)-D -O-2,5-anhydro [1-³H]mannitol-6-sulphate, GlcNAc6S-IdoA2S-anM6S, which were less than 1 per cent of the normal range for fibroblasts cultured in Basal Eagle's medium supplemented with fetal calf serum. However, the glucosamine-6-sulphatase activities of MPS IIID fibroblasts grown in Chang's medium were similar to the activities in normal control fibroblasts which were cultured in Basal Eagle's medium. These results indicate that caution is required for prenatal diagnosis of MPS IIID patients using chorionic villi or amniotic cells cultured in Chang's medium.     

Technical and theoretical considerations in the HLA typing of amniotic fluid cells for prenatal diagnosis and paternity testing

HLA typing of amniotic fluid cells has been used for the prenatal diagnosis of the HLA linked diseases congenital adrenal hyperplasia (21-OH-deficiency (21-OH-def) type) and complement C4 deficiency and it has also been used for the prenatal de termination of paternity. There are, however, technical difficulties in this test associated with the weak expression of some B locus antigens on amniotic fluid cells, and theoretical difficulties related to associations between particular HLA antigens and the 21-OH-def allele. Since certain HLA-B locus antigens are found in significantly increased frequencies among patients with 21-OH-def, there is a relatively high incidence of HLA-B homozygosity among the patients and over 40 percent of the parents of these patients share one or more HLA-B locus antigens. Results of some prenatal HLA typing tests may thus be difficult to interpret, and supplementary tests should be used whenever possible. HLA typing of amniotic cells is, however, the only available procedure for prenatal diagnosis of C4 deficiency and it is the best available procedure for prenatal determination of paternity. A modification of our original procedure allows HLA typing to be performed with increased numbers of HLA typing sera, and sera with optimum reactivity for amniotic fluid cells have now been selected for the definition of most of the more commonly expressed HLA antigens. Although amniotic fluid cells do not express DR antigens, amniotic fluid cells can be typed for the HLA-linked marker glyoxalase I (GLO) and this may be the informative for prenatal diagnosis in some cases.     

SF6绝缘设备的环保与经济运行维护

SF6气体作为一种优秀灭弧介质在高压灭弧领域得到了十分广泛的应用.同时,SF6气体的一些弊端,如泄漏对人体与环境的影响也体现出来,通过介绍SF6气体的环保要求和运行、检测、维护及未来的发展方向.使得其在环境保护的前提下,在电力系统得到更加广泛的应用.     

电视机,电脑视屏与β辐射的关系

对电视机，电脑视视屏显象管壳成分进行了β放射性活性度测定及β剂量的计算，结果证明，视屏的固有电离辐射－β射线来自玻璃原材料，所产生的β剂蜈取决于原材料成分中天然放射性核素的种类含量及所用的份额。由此产生的β剂量对公众的健康不会产生有害影响。     

First-trimester prenatal diagnosis of Sanfilippo C disease

At 10 weeks' gestation, chorionic villus biopsy was obtained from a woman at risk for Sanfilippo type C disease. Acetyl-CoA: α-glucosaminide N-acetyltransferase activity was markedly deficient. The pregnancy was terminated at 12 weeks' gestation and follow-up study on fetal fibroblasts confirmed the diagnosis.     

Carbon isotope ratios in logged and unlogged boreal forests: Examination of the potential for determining wildlife habitat use

Due to assimilation of recycled CO₂ from litter decomposition and photosynthetic changes in carbon fractionation at low light levels, the foliage at the base of a forest is often more depleted in¹³C compared to that exposed to the atmosphere in either the canopy or in open clearings. This is referred to as the canopy effect. African research has indicated that these habitat differences in foliar ¹³C can be substantial enough to affect the carbon isotope ratios of resident fauna. Previous work documenting a 30-year chronology on moose teeth from Isle Royale National Park indicated a progressive depletion in¹³C and suggested that this could be due to forest regrowth following extensive burning. The present study examined the assumption implicit in this hypothesis that foliar ¹³C varies between open and closed boreal forest sites. I found a marginal canopy effect of 2 ¹³C difference between upper canopy and ground flora for a forest in northwestern Ontario and an average difference of 1.2 in under- and mid-story vegetation between closed forests and open clear-cuts. Because of these small differences, the utility of carbon isotope analysis in quantifying temporally integrated exploitation of deforested habitats will be low for northern boreal locations. In denser forests, such as those in the tropics or western North American where the canopy effect can be expected to be much greater, ¹³C analysis may still offer some promise for determining selection by wildlife of disturbed habitats.     

Characterisation of Organic Matter and Carbon Cycling in Rehabilitated Lignite-rich Mine Soils

Open-cast lignite mining in the Lusatian mining district resulted in rehabilitated mine soils containing up to four organic matter types: (1) recent plant litter, (2) lignite deposited by mining activity, (3) carbonaceous ash particles deposited during amelioration of the lignite-containing parent substrate and (4) airborne carbonaceous particles deposited during contamination. The influence of lignite-derived carbon types on the organic matter development and their role in the soil carbon cycle was unknown. This paper presents the findings obtained during a six year project concerning the impact of lignite on soil organic matter composition and the biogeochemical functioning of the ecosystem. The organic matter development after rehabilitation was followed in a chronosequence of rehabilitated mine soils afforested in 1966, 1981 and 1987. A differentiation of the organic matter types and an evaluation of their role within the ecosystem was achieved by the use of ¹⁴C activity measurements, ¹³C CPMAS NMR spectroscopy and wet chemical analysis of plant litter compounds. The results showed that the amount and degree of decomposition of the recent organic matter derived from plant material of the 30 year old mine soil was similar to natural uncontaminated forest soil which suggests complete rehabilitation of the ecosystem. The decomposition and humification processes were not influenced by the presence of lignite. On the other hand it was shown that lignite, which was thought to be recalcitrant because of its chemical structure, was part of the carbon cycle in these soils. This demonstrates the need to elucidate further the stabilisation mechanisms of organic matter in soils.     

真,假草本咖啡种子的鉴别

在对草本咖啡（Ｃａｓｓｉａｓｏｐｎｅｒａｉｉｎｎ）、望江南（ＣａｓｓｉｓｏｃｃｉｄｅｎｔａｌｉｏＬ．）和决明（ＣａｓｓｉａｔｏｒａＬ．）种子的大小、颜色、形态进行了观察、研究，并对其形态特征进行了解剖学的分析后，提出了鉴别３种种子的方法。     

电化学法测定粮食中铜的含量

在0.02mo1/L的六次甲基四胺(C6H12N4)缓冲液(pH 5.5)中,铜(Ⅱ)—6-苄基氨基嘌呤(6-BA)在KNO3存在下,于-0.50V左右(vs,SCE)产生一个尖锐、灵敏的二次导数极谱波,峰电流与铜(Ⅱ)浓度在1.0×10-5~3.0×10-7mol.L-1范围内呈线性关系,检出限为1×10-7mol.L-1。该方法用于粮食中铜的测定,结果满意。     

青藏铁路救援指挥系统的研究

通过对青藏铁路特点及运营模式的分析,提出构建青藏铁路事故救援指挥系统的重要性。结合青藏铁路信息化总体规划,在充分利用青藏铁路各信息系统及通讯网络基础上,利用地理信息系统(GIS)技术和无线通讯技术,建设具有指挥、控制、通信及情报(C3I)功能的青藏铁路事故救援指挥系统。提出了系统应达到的目标功能,结合青藏铁路生产作业实际情况,给出了系统实现的信息流程、体系结构,并开发了原型系统。系统将青藏铁路多源信息整合并结合GIS技术以直观的电子地图、图像等方式展示出来,实现了救援预案管理和智能化查询、匹配;并通过应急无线通讯设备实现事故现场视频、图像实时播放及指挥调度与事故现场间的语音双向通讯;同时可建立事故数据库,为统计报告和事故分析服务。